The onset of the disease is usually before age 2, but patients have been diagnosed with PFIC even into adolescence. Of the three entities, PFIC-3 usually presents earliest. Patients usually present in early childhood with cholestasis, jaundice, and failure to thrive. Intense pruritus is characteristic; in patients who present in adolescence, it has been linked with suicide. Patients may have fat malabsorption, leading to fat soluble vitamin deficiency, and complications, including osteopenia.

Progressive familial intrahepatic cholestasis (PFIC) is a group of familial cholestatic conditions caused by defects in biliary epithelial transporters. The clinical presentation usually occurs first in childhood with progressive cholestasis. This usually leads to failure to thrive, cirrhosis, and the need for liver transplantation.

Most women with this condition present in third trimester with itching without a rash. Typically, the itching is localized to the palms of the hands and soles of the feet but can be anywhere on the body.

Hallmarks of ICP include the following symptoms:

Most common:

- Itching, in particular but not limited to that of the palms of the hands and soles of the feet, without presence of a rash

- Itching that increases in the evening

- Itching that does not respond favorably to anti-histamines or other anti-itch remedies

- Often, elevated LFT results as well as serum bile acid counts

Less common:

- Darker urine

- Lighter stools

- Increased clotting time (due to possibly associated vitamin K deficiency)

- Fatigue

- Increased nausea

- Decrease in appetite

- Jaundice

- Upper right quadrant pain

It is important to note that not all ICP sufferers have all of the above symptoms. For example, Jaundice only occurs in relatively small subset of cases, and in some cases abnormal lab results were not seen until 15 weeks or more after the onset of symptoms.

Intrahepatic cholestasis of pregnancy (ICP), also known as obstetric cholestasis, cholestasis of pregnancy, jaundice of pregnancy, and prurigo gravidarum, is a medical condition in which cholestasis occurs during pregnancy. It typically presents with troublesome itching and can lead to complications for both mother and fetus.

Pruritus (itching) has long been considered to be a common symptom of pregnancy. The vast majority of times, itching is a minor annoyance caused by changes to the skin, especially that of the abdomen. However, there are instances when itching is a symptom of ICP. This is usually most intense on the palms of the hands, and the soles of the feet, but can be widespread.

ICP occurs most commonly in the third trimester, but can begin at any time during the pregnancy.